RESUMEN
EBV+ inflammatory follicular dendritic cell (FDC) sarcoma is an indolent malignant neoplasm of spindled FDCs with a rich lymphoplasmacytic infiltrate and a consistent association with Epstein-Barr virus (EBV). It occurs exclusively in the liver and spleen, with the exception of a few colonic examples. In this study, we report 9 extrahepatosplenic cases, including 4 occurring in previously undescribed sites, but all apparently anatomically related to the aerodigestive tract. The cases included 5 gastrointestinal tumors all presenting as colonic pedunculated polyps, 2 presenting as mesocolon mass, and 2 involving the palatine or nasopharyngeal tonsils. One patient with a colonic tumor was complicated by paraneoplastic pemphigus. The patients had a median age of 58 years, with female predominance (female:male=7:2). A favorable outcome was observed in 7 patients. Histologically, EBV+ inflammatory FDC sarcomas arising from these anatomic sites were similar to their hepatosplenic counterparts. Spindled to oval neoplastic cells with ill-defined cell borders were dispersed or formed loose whorled fascicles in a dense lymphoplasmacytic background. They had vesicular nuclei with distinct nucleoli and typically exhibited a range of nuclear atypia in the same case. The neoplastic cells showed variable expression of FDC markers and were labeled for Epstein-Barr virus-encoded RNA on in situ hybridization. These 9 cases thus broaden the clinicopathologic scenarios of EBV+ inflammatory FDC sarcoma. Recognition of the potential existence of this tumor type in extrahepatosplenic sites permits a correct diagnosis to be made.
Asunto(s)
Neoplasias del Colon/patología , Pólipos del Colon/patología , Sarcoma de Células Dendríticas Foliculares/patología , Infecciones por Virus de Epstein-Barr/virología , Herpesvirus Humano 4/genética , ARN Viral/genética , Neoplasias Tonsilares/patología , Adulto , Anciano , Biomarcadores de Tumor/análisis , Niño , Neoplasias del Colon/química , Neoplasias del Colon/cirugía , Neoplasias del Colon/virología , Pólipos del Colon/química , Pólipos del Colon/cirugía , Pólipos del Colon/virología , Sarcoma de Células Dendríticas Foliculares/metabolismo , Sarcoma de Células Dendríticas Foliculares/cirugía , Sarcoma de Células Dendríticas Foliculares/virología , Femenino , Humanos , Inmunohistoquímica , Hibridación in Situ , Masculino , Persona de Mediana Edad , Neoplasias Tonsilares/química , Neoplasias Tonsilares/cirugía , Neoplasias Tonsilares/virologíaRESUMEN
We present an unusual case of rapid onset of vitiligo heralding blindness due to pituitary apoplexy secondary to a non-secreting pituitary adenoma in a 49-year-old man. Deterioration in vision and skin condition coincided with tumor progression over 3 years. He subsequently died of acute lymphoblastic leukemia after pituitary radiation. The possible pathological associations between the 2 malignancies and vitiligo are discussed.
